Esophageal Manometric Abnormalities in Parkinson's Disease

The gastrointestinal tract, and especially the esophagus, is frequently involved in neurological diseases; however, objective studies of gut motor function are few. We carried out an esophageal manometric study in 18 patients with various stages of Parkinson's disease (4 stage I, 4 stage II, 7 stage III, and 3 stage IV) to evaluate the function of the viscus in this disease. Clinical assessment showed that 61% complained of esophageal symptoms such as dysphagia, acid regurgitation, pyrosis, and noncardiac chest pain. Manometric abnormalities were documented also in 61% patients, and were represented by repetitive contractions, simultaneous contractions, reduced LES pressure, and high-amplitude contractions. However, only 33.3% of patients had both symptoms and manometric abnormalities. We conclude that esophageal motor abnormalities are frequent in Parkinson's disease, and may appear at an early stage of the disease.     

Transition from peristaltic esophageal contractions to diffuse esophageal spasm

A patient with dysphagia and chest pain was shown by manometry to have high-amplitude peristaltic esophageal contractions (nutcracker esophagus). Worsening symptoms over the next two years led to the performance of repeated manometric studies, which showed diffuse esophageal spasm. This demonstration of a transition from nutcracker esophagus to diffuse esophageal spasm lends further support for consideration of the nutcracker esophagus as a manometric disorder associated with chest pain or dysphagia. Furthermore, it suggests a pathophysiologic relationship between the nutcracker esophagus, a disorder with preserved peristalsis, and diffuse esophageal spasm, the classic dysmotility considered to be of neurogenic origin.     

Nutcracker esophagus: clinical evaluation of 97 patients]

Nutcracker esophagus is a manometric abnormality classified as a primary esophageal motor disorder, characterized by high pressure peristaltic waves in distal esophagus and related to non-cardiac chest pain. Further studies observed nutcracker esophagus in dysphagic patients and recently in gastroesophageal reflux disease. However, there is controversy about the meaning of this motor disorder and there are few clinical studies involving a great number of patients. A retrospective study involving 97 patients with manometric criteria of nutcracker esophagus according a control group was undertaken. Most of the patients were female (63.9%), mean age 54.3 years. The chief complaint was chest pain, followed by dysphagia and heartburn. Clinical findings, as a whole were chest pain (53.6%), dysphagia (52.6%), heartburn (52.6%), regurgitation (21.6%), otorhinolaryngologic symptoms (15.4%), dyspepsia (15.4%) and odynophagia (4.1%). The majority of patients had multiple symptoms, however in 28% just a single one was observed. Endoscopic examination observed erosive esophagitis in 8% of the patients, while signs of esophageal motor disorders were showed by esophagogram in 16.4%. Esophageal pH recordings indicated abnormal gastroesophageal reflux in 41.2% of the cases reported. We concluded that there are other symptoms in nutcracker esophagus patients besides chest pain and dysphagia and the use of esophageal pH recordings is helpful to establish its association with acid reflux and guide the appropriate therapy.     

Dysphagia in Patients with Chagas' Disease

Some patients with Chagas' disease and apparent normal esophageal function complain of dysphagia. With the objective of investigating the esophageal motility of these patients we studied the esophageal contraction amplitude, duration, velocity, and lower esophageal sphincter (LES) pressure of 34 patients with a positive serologic test for Chagas' disease, normal radiologic esophageal examination, peristaltic contractions in the esophageal body, and complete LES relaxation. Fourteen patients complained of dysphagia and 20 had no symptoms. The results were compared with those of 22 healthy controls. We used the manometric method with continuous perfusion. In patients without dysphagia, the LES pressure (17.8 ± 1.2 mmHg, mean ± SEM) and distal esophageal amplitude (71.8 ± 7.9 mmHg) were lower than those of control subjects (24.3 ± 1.8 mmHg and 100.4 ± 10.6 mmHg, respectively). The velocity of peristaltic contractions was higher in patients than in controls, but there was no difference between patients with or without dysphagia. The duration of contraction in the distal esophagus was longer in patients with dysphagia (3.9 ± 0.2 sec) than in patients without dysphagia (3.1 ± 0.2 sec) and controls (3.2 ± 0.2 sec). We conclude that dysphagia in patients with Chagas' disease and a nondilated esophagus with peristaltic contractions and complete LES relaxation is related to a longer duration of contractions in the middle and distal esophageal body.     

"Segmental Aperistalsis" of the Esophagus: A Cause of Chest Pain and Dysphagia

Although some patients with chest pain and dysphagia have manometric evidence of classic esophageal motor disorders, other patients with these symptoms may have only nonspecific findings of unknown importance. We describe five patients with chest pain and dysphagia in whom esophageal manometry showed a segment of esophagus with an increased frequency of simultaneous contractions associated with normal motility in the more proximal and distal esophagus. All patients had corresponding segmental abnormalities on video-esophagograms augmented with a solid holus; in four patients, the solid bolus caused reproduction of symptoms during the esophagography. We conclude that "segmental aperistalsis" may cause chest pain and dysphagia, and that the diagnosis may be made by careful manometric analysis of the entire esophagus, complemented by esophagography with a solid bolus.     

Surgical myotomy in patients with high-amplitude peristaltic esophageal contractions

High-amplitude peristaltic esophageal contractions, or the nutcracker esophagus, may be associated with chest pain or dysphagia. Medical treatment for this disorder is sometimes not satisfactory. We report the manometric and clinical effects of myotomy in four patients with high-amplitude peristaltic contractions who underwent surgery because of the severity of their symptoms and recalcitrance to various medical treatments. Manometry 1-5 years after surgery showed a reduction in amplitude, duration, and percent bipeaked waves at 5 and 10 cm above the lower esophageal sphincter. Peristalsis was abolished or decreased in the distal 10 cm of the esophageal body but was not affected more proximally. Lower esophageal sphincter pressure was decreased in all patients. The manometric changes were least marked in one patient, who was the only one who had some chest pain when last seen five years after myotomy. We conclude that in severely symptomatic patients with high-amplitude peristaltic contractions, myotomy results in marked manometric changes and marked clinical improvement. Patients with this disorder and whose chest pain is recalcitrant to extensive medical therapy may be successfully treated by surgical myotomy.     

Segmental High Amplitude Peristaltic Contractions in the Distal Esophagus

High amplitude peristaltic contractions in the distal esophagus ("nutcracker esophagus") is the most common manometric disorder seen in patients with noncardiac chest pain. Although this abnormality is found in the distal esophagus, the definition regarding its precise level in the esophagus is unclear. A careful analysis of 99 consecutive manometric tracings performed during a 1-yr period revealed that in patients with noncardiac chest pain and/or dysphagia, the location of the abnormal esophageal contractions varied: 1) in 11 patients the esophageal contractions were abnormal at 2 cm, as well as 7 cm, above the lower esophageal sphincter (LES); 2) the abnormality was limited to the 2-cm location above the LES in six patients; and 3) was confined to the 7-cm location above the LES in five patients. If the conventional criteria of averaging the distal esophageal contraction amplitudes at 2 and 7 cm above the LES were adopted, six of the 11 patients with segmental esophageal contraction abnormality would not have been identified. We suggest that, by inspection of each location of the distal esophagus separately, localized high amplitude contractions can be identified, and the distal 2 cm segment of the esophagus should be routinely included in the manometric evaluation.     

A Questionnaire Study to Assess Long-Term Outcome in Patients with Abnormal Esophageal Manometry

Patients with unexplained chest pain or dysphagia are often referred for esophageal manometric studies to further investigate their symptoms. Four main manometric abnormalities have been described: achalasia, diffuse esophageal spasm, “nutcracker” (hypercontracting) esophagus, and hypocontracting esophagus. With the exception of achalasia, treatments are of limited benefit and the natural history of these conditions is largely unknown. We sent questionnaires to patients who were investigated at least three years before our study began. They repeated a DeMeester symptom questionnaire that they had completed at the time of their initial study. Questionnaires were sent to 137 patients with diffuse esophageal spasm, “nutcracker” (hypercontracting) esophagus, or hypocontracting esophagus. We also sent questionnaires to 57 patients with dysphagia or chest pain who had had normal esophageal manometry and pH studies. These patients acted as symptomatic controls. Responses were compared using the Wilcoxon signed ranks test. Seventy-two (53%) patients with diffuse esophageal spasm, “nutcracker” esophagus, or hypocontracting esophagus replied. An additional 8 (6%) patients died. Symptom scores in all three conditions had improved significantly over time (p ≤ 0.01 for each condition, Wilcoxon signed ranks test). Patients with dysphagia or chest pain but normal esophageal studies had not improved. The significance of diffuse esophageal spasm, “nutcracker” esophagus, and hypocontracting esophagus found at esophageal manometry remains uncertain. Although treatment is often ineffective, these conditions typically run a benign course. Patients can be reassured that their symptoms are likely to improve with time.     

Transition from nutcracker esophagus to achalasia

Summary Nutcracker esophagus is essentially a manometric diagnosis characterized by high-amplitude, often prolonged duration of peristaltic contractions in the distal two thirds of the esophagus. Its association with noncardiac chest pain and/or dysphagia has been recognized and reported by numerous esophageal motility laboratories. There are very few long-term studies of the natural history of this abnormality. We report a patient who presented with dysphagia and, on initial investigation, was found to have classical nutcracker esophagus. On reinvestigation three years later, however, he had developed achalasia of the cardia. The transition from nutcracker esophagus to achalasia has not previously been reported.     

Esophageal motor disorders in patients evaluated for dysphagia and/or noncardiac chest pain

During the period January, 1983–October, 1990, 429 subjects were referred for functional evaluation of dysphagia and/or noncardiac chest pain. Of these, 304 (70.8%) were shown to have some kind of esophageal motor abnormality. The most frequent motor abnormality of the esophagus was represented by nonspecific motor disorders (31%), followed by achalasia (13%), whereas the other dysfunctions accounted for a smaller percentage. In particular, diffuse esophageal spasm was shown to be quite rare. It is concluded that esophageal manometry may provide a high diagnostic yield in patients presenting with dysphagia and/or noncardiac chest pain.     

Segmental versus Diffuse Nutcracker Esophagus: An Intermittent Motility Pattern

The most common esophageal motility abnormality in patients with noncardiac chest pain is nutcracker esophagus. Most investigators regard nutcracker esophagus as a diffuse process involving the distal esophagus. Others consider it a segmental disturbance affecting isolated regions of the distal esophageal smooth muscle. This study compared the prevalence, clinical features, consistency, and manometric course of patients with either segmental high-amplitude peristaltic contractions (SHAPC) or those with the traditional diffuse contraction abnormalities termed nutcracker esophagus (NE). We particularly sought to determine whether patients with SHAPC represent an early spectrum evolving into a more diffuse contraction disorder—NE. The prevalence and clinical features of patients with either motility disturbance were similar. Thirty-nine percent of our patients had abnormally high peristaltic amplitude in locations of the proximal esophagus not previously described. Follow-up manometric studies demonstrated that only 53% of patients with NE and 20% with SHAPC retained the same manometric diagnosis. In addition, 33% of patients in the NE group and 40% of the SHAPC group permutated into each other. These findings indicate that patients with SHAPC do not represent an early process subsequently evolving into a more diffuse contraction abnormality. Rather, the motility pattern of high-amplitude peristaltic contractions—segmental or diffuse—constitutes a labile marker associated with noncardiac chest pain.     

Diltiazem Therapy for Symptoms Associated with Nutcracker Esophagus

A randomized double-blind, cross-over prospective trial in 22 patients was designed to evaluate possible effect of an oral calcium channel blocker, diltiazem, on symptoms of chest pain and/or dysphagia in patients with nutcracker esophagus. We studied 22 consecutive patients referred to an esophageal diagnostic center for evaluation of noncardiac chest pain or dysphagia having high amplitude esophageal contractions, 14 of whom completed the study. Diltiazem (60-90 mg qid) was compared with placebo, each being administered for 8 wk. Patients were evaluated with esophageal motility pre- and posttreatment periods and with regular symptom assessment throughout each 8-wk treatment. Active diltiazem therapy resulted in significantly lower (p less than 0.05) mean distal esophageal peristaltic pressure (128 +/- 20 mm Hg; +/- SE) than placebo (158 +/- 16 mm Hg). Mean chest pains scores were significantly (p less than 0.05) lower with diltiazem therapy than with placebo. Only nine of the 14 patients fulfilled presently acceptable criteria for diagnosing nutcracker esophagus, and the diltiazem effect was similar, although not significant, because of the smaller sample. Conclusions: In this preliminary study involving 14 patients, the oral calcium channel blocker, diltiazem, appeared to improve noncardiac chest pain associated with strong esophageal contraction, the nutcracker esophagus. These improved symptoms were associated with significant decreases in contraction pressure.     

Manometric and Radiologic Correlations in Achalasia

Achalasia is an esophageal motor disorder distinguished by clinical, radiologic, and manometric features. To evaluate the correlation among these features, we studied 109 achalasia patients. The four most common clinical complaints, the four most commonly encountered radiologic findings, and two manometric parameters were analyzed with a correlation matrix test and a multiple regression analysis. Significant correlation existed among symptoms of dysphagia, regurgitation, and weight loss. In contrast, chest pain inversely correlated with these symptoms. Dysphagia and weight loss significantly correlated with a bird-beak deformity but not with esophageal dilatation or a sigmoid esophagus. Moreover, no significant relationship between lower esophageal sphincter pressure and esophageal dilatation or sphincter pressure and sigmoid esophagus was found. However, in those patients with a resting lower esophageal sphincter pressure greater than 45 mm Hg, a reasonable correlation among clinical, radiologic, and manometric parameters did exist. In conclusion, although in a subset of patients with markedly increased lower esophageal sphincter pressure, a good correlation between clinical, radiologic, and manometric findings exists, such a correlation cannot be established in all of the achalasia patients; esophageal dilatation or a sigmoid esophagus may not be due to a hypertensive sphincter, and their presence must not necessarily be interpreted as an indication of severity of the disease; there is an inverse correlation between chest pain and symptoms of dysphagia, regurgitation, and weight loss; and finally, achalasia and hiatal hernia may coexist in 6% of the patients.     

Chest Pain—Esophageal, Cardiac, or Both?

The esophagus may be the origin of chest pain clinically indistinguishable from that of ischemic heart disease. In some patients the esophageal origin of the pain may only be recognized by pharmacological provocation during manometry. We describe nine patients with chest pain which could be explained by disorders of esophageal motility--diffuse spasm in four, high pressure lower esophageal sphincter in three, and "nutcracker esophagus" in two. Methacholine provoked the pain and manometric abnormalities in five patients who had normal baseline tracings. However, seven patients given methacholine developed ischemic changes on the electrocardiogram. In one patient these were typical of Prinzmetal's variant angina. The manometric and electrocardiographic abnormalities were reversed by intravenous atropine. Ischemic heart disease and esophageal motor disorders may occur concomitantly and pose a dilemma in diagnosis and management.     

Esophageal testing of patients with noncardiac chest pain or dysphagia. Results of three years' experience with 1161 patients

Records from 910 patients referred to our clinical esophageal manometry laboratory for evaluation of noncardiac chest pain between January 1983 and December 1985 were reviewed and compared with records from 251 patients referred for dysphagia. Evaluation included baseline esophageal manometry, acid perfusion test, and edrophonium provocation. In the chest-pain group, 655 patients (72%) had normal esophageal motility and 255 (28%) had abnormal motility. Nutcracker esophagus was present in 48% of abnormal tracings, suggesting that it is a manometric marker for noncardiac chest pain. Of the total chest-pain group, 243 patients (27%) had their pain reproduced during provocative testing ("definite" esophageal pain); 192 patients (21%) had baseline manometric abnormalities but no pain during provocative testing ("probable" esophageal chest pain). The highest percentage of positive provocative responses (34%) occurred in patients with nutcracker esophagus on baseline manometry. Manometric abnormalities were statistically commoner (p less than 0.001) in patients with dysphagia, occurring in 53%. Achalasia (36%) and nonspecific esophageal motility disorders (38%) were the commonest abnormalities in this group, with nutcracker esophagus being infrequent (10%).     

Nutcracker esophagus: GERD or an esophageal motility disorder

A retrospective study was performed to determine the frequency of acid-related esophageal dysfunction in an unselected group of patients with nutcracker esophagus (NE). Five hundred seventy-two consecutive patients who underwent esophageal manometry and pH testing at one institution were evaluated. Forty-one percent were referred for evaluation of chest pain, 39% for reflux symptoms, and 20% for dysphagia, nausea, or epigastric pain. Esophageal manometry and 24-h pH monitoring were performed using standard methods. NE was defined as amplitude of phasic contractions of ≥180 mm Hg in any manometric tracing at any level of the esophagus. Abnormal total reflux was defined as >4% of the time with the esophageal pH < 4. A positive symptom index was defined as >50% of periods with pH < 4 coinciding with symptoms of chest pain or heartburn. Esophagitis was defined as an unequivocal mucosal defect if esophagogastroduodenoscopy was performed.
Forty-five patients met criteria for NE, with acid-related abnormalities found in 77%. Forty-nine percent had abnormal acid exposure time, 16% had positive symptom indexes with normal acid exposure, and 5% had endoscopic esophagitis. An additional 7% had only an increased number of reflux episodes with normal acid exposure and symptom indexes. The prevalence of NE was significantly higher in patients referred for chest pain than for typical reflux symptoms (14.3% vs 4.5%). Seventy-four percent of the patients with NE and chest pain did not have classic reflux symptoms. Seventy-six percent of 34 evaluable subjects who had been started on acid suppression were either improved or symptom free at an average of 10.7 months of follow-up.     

The Changing Faces of the Nutcracker Esophagus

Although the nutcracker esophagus, characterized by high amplitude peristaltic contractions with mean distal amplitude greater than 180 mm Hg, is the most common esophageal motility disorder associated with noncardiac chest pain, little is known about its natural history. Therefore, we reviewed the manometric tracings of 23 patients with the nutcracker esophagus who had an average of 4.6 studies during a mean period of 32 months. Ten age-matched volunteers with normal baseline manometry who had undergone multiple studies (mean 5.8) over a mean time span of 32 months served as controls. In the 17 nutcracker patients with three or more motility studies, the variability of mean distal amplitudes between studies was 41.9% +/- 4.1 (+/- SE) compared to 27.0% +/- 3.3 for the control subjects (p less than 0.01). Highest distal pressures were noted during the first study in 11 of 17 patients (65%) compared to two of 10 controls (20%). The consistency of the diagnosis of nutcracker esophagus varied considerably: four patients always had high amplitude pressures, three patients only had the nutcracker diagnosis on the initial study, and 10 patients intermittently had pressures in the nutcracker range. Overall, these 17 patients had the diagnosis of the nutcracker esophagus confirmed on only 54% of subsequent studies. Changes in motility patterns were intermittently seen in six of 23 patients: one diffuse spasm and five nonspecific motility disorders. None of the control subjects developed high amplitude contractions or changed their motility pattern on serial testing. The possible pathophysiological implications of the changing faces of the nutcracker esophagus are discussed.     

Diagnosis of chest pain with foregut symptoms in Chinese patients

AIM： To evaluate the diagnosis of chest pain with foregut symptoms in Chinese patients.
METHODS： Esophageal manometric studies, 24-h introesophageal pH monitoring and 24-h electrocardiograms （Holter electrocardiography） were performed in 61 patients with chest pain.
RESULTS： Thirty-nine patients were diagnosed with non-specific esophageal motility disorders （29 patients with abnormal gastroesophageal reflux and eight patients with myocardial ischemia）. Five patients had diffuse spasm of the esophagus plus abnormal gastroesophageal reflux （two patients had concomitant myocardial ischemia）, and one patient was diagnosed with nutcracker esophagus.
CONCLUSION： The esophageal manometric studies, 24-h intra-esophageal pH monitoring and Holter electrocardiography are significant for the differential diagnosis of chest pain, particularly in patients with foregut symptoms. In cases of esophageal motility disorders, pathological gastroesophageal reflux may be a major cause of chest pain with non-specific esophageal motility disorders. Spasm of the esophageal smooth muscle might affect the heart-coronary smooth muscle, leading to myocardial ischemia.     

Unexplained dysphagia: Viscous swallow-induced esophageal dysmotility

Dysphagia is a manifestation of several clinical conditions of diverse origin. In spite of the variation in these disease entities in terms of their etiology, clinical presentation, natural history, and treatment, the mechanism of this clinical complaint is not always clear. We studied a group of patients with dysphagia for solids in whom no anatomic or motor abnormalities were encountered on standard studies. The group consisted of 37 patients, 25 women and 12 men, who were complaining of dysphagia of 6 months or longer duration and they did not demonstrate structural or motor abnormalities on barium esophagogram, esophagoscopy, and standard esophageal manometry. A group of 24 age-matched patients, 14 women and 10 men, with noncardiac chest pain served as the patient control. Esophageal contractile activities were studied after 10 wet swallows (5 ml of water) and 10 viscous swallows (5 cubic cm of marshmallow). Resting lower esophageal sphincter pressure and its relaxation response to swallows, amplitude of peristaltic activities, rate of dysphagia provoked during the study, and the frequency of abnormal esophageal contractions were evaluated. Six abnormal esophageal contractile activities—failed peristalsis, dropout, repetitive, simultaneous, spontaneous contractions, and aperistalsis—were utilized to generate an esophageal peristaltic dysfunction index. The mean LESP was 8.1±4.7 in the dysphagia group and 16.1±4.3 in the chest pain group. The mean amplitude of peristaltic contractions was 47.1±16.1 and 89.0±27.0 mmHg after wet swallows for dysphagia and chest pain groups, respectively. These values were 58.2±12.4 and 92.4±22.1 for viscous swallows. Swallowing provoked dysphagia in 89% of the dysphagia group after viscous swallows and 9% after wet swallows. In contrast, only 11% and 3% of control group complained of dysphagia during the study. This group of patients probably represent a cohort of patients with a nonspecific esophageal motor disorder in whom both clinical symptom and their esophageal motor counterpart can only be elicited in response to viscous swallows. We strongly believe in addition of viscous swallows in evaluating dysphagic patients in whom symptoms remain unexplained in light of standard studies.     

Clinical and Radiologic Evaluation Reveals High Prevalence of Abnormalities in Young Adults with Dysphagia

The purpose of this study was to retrospectively evaluate the radiologic findings in young adults with dysphagia undergoing barium swallow and to compare these with the final clinical diagnosis. Clinical history, barium swallow, endoscopy (21 patients), manometry (18 patients), 24 h pH monitoring (4 patients), and outcome of treatments were studied and compared in 43 patients aged 14–30 years (mean 24 years). There were 26 men and 17 women. Duration of symptoms varied between 2 weeks and 22 years and included globus (n = 22), obstruction (n = 31), water brash (n = 6), classic reflux symptoms (n = 10), atypical reflux symptoms (n = 9), slow eating (n = 6), and vomiting (n = 11). The final diagnosis was achalasia (n = 2), arteria lusoria (n = 1), esophagitis (n = 1), esophageal dysfunction (n = 11), esophageal stricture (n = 5), gastroesophageal reflux disease (n = 8), and pharyngeal dysfunction (n = 2). Thirteen patients were assessed to be normal. The result of the barium swallow was in agreement with the final diagnosis in all but 3 patients who were assessed as normal, and the final diagnosis was esophagitis (n = 1), dysmotility (n = 1), and reflux disease (n = 1). Anatomic and functional abnormalities are common in young adults with dysphagia. Barium swallow reveals the explanation of the symptoms in 70% of such patients. Radiology therefore should be the method of choice for the investigation of dysphagic young adults.